i(3)so3-galactosylceramide and Leukoencephalopathy--Progressive-Multifocal

A study was conducted on the fatty acid composition of cerebroside, sulfatide and ganglioside in the brains of 4 cases of Nasu-Hakola disease. The percentage of short carbon chain (C.16-C.18) nonhydroxy fatty acids of sulfatide in the cortex was much higher than that of the control and long-chain (C.24-) fatty acids showed a lower percentage. Sulfatide in the white matter was of the same tendency. The percentage of C.16:0 palmitic acid of ganglioside in the cortex was much larger than that of the control. Referring to the fatty acid composition of lipids in the literature, abnormalities of the fatty acid concentration, namely a high percentage of short-chain fatty acids of sulfatide and ganglioside in the cerebral cortex of the present cases, were confirmed.

Topics: Adult; Axons; Cerebral Cortex; Cerebrosides; Dementia; Fatty Acids; Fatty Acids, Volatile; Female; Galactolipids; Gangliosides; Glycolipids; Humans; Inclusion Bodies; Leukoencephalopathy, Progressive Multifocal; Lipodystrophy; Male; Middle Aged; Myelin Sheath; Sulfoglycosphingolipids